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Airflow Obstruction and Dysphonia in a Nonsmoker

Case Editor - Judd Flesch

Reviewed By Clinical Problems Assembly

Submitted by

Albert James Mamary, MD

Assistant Professor of Medicine

Temple University School of Medicine

Philadelphia, Pennsylvania

Alex E. Swift, MD

Fellow, Pulmonary and Critical Care Medicine

Temple University School of Medicine

Philadelphia, Pennsylvania

Gilbert E. D'Alonzo, DO

Professor of Medicine

Temple University School of Medicine

Philadelphia, Pennsylvania

Submit your comments to the author(s).

History

The patient is a 62-year-old Caucasian woman who is seeking consultation for progressive dyspnea on exertion.  She can walk a few city blocks at a leisurely pace and a single flight of stairs but no longer participates in fitness activities.  She was previously diagnosed with COPD but had never smoked or lived with a smoker.  Her dyspnea gradually began 5 years ago.  She has no history of asthma. She has noticed very rare wheezing, a minimal nonproductive cough, but no chest tightness.  Previous treatments have included oral and inhaled corticosteroids, and immediate- and long-acting beta2 agonists and anticholinergic inhalers.  She did not experience a change in her dyspnea or spirometry with any medication. Twenty-five years ago she was hit in the neck with a batted baseball.  A few years later she developed pain in her hands and wrists and episodic dysphonia. Her dysphonia worsened and culminated in severe stridor and acute respiratory distress, necessitating an emergent tracheostomy and surgical fixation of the left vocal cord.  She attributes these problems to the baseball accident. Her joint pain has persisted and is associated with swelling and stiffness.

Past Medical and Surgical History: left vocal cord paralysis, dysphonia, COPD, tracheostomy, hypothyroidism, gastroesophageal reflux, and three uncomplicated vaginal deliveries. There is no history of asthma, allergic rhinitis, ocular disease, hepatorenal diseases, circulatory diseases, gastrointestinal problems or diabetes mellitus.

Allergies: none  

Medications: daily use of inhaled tiotropium and oral levothyroxine, esomeprazole, and naproxen.

Family History: mother died of ovarian cancer and father died of complications associated with Alzheimer’s dementia.  She has three adult children who are in good health. There is no family history of lung disease.

Social History: negative for tobacco, alcohol or illicit drug use. She works as a secretary and lives with her husband who is a schoolteacher in the suburbs of a large city.

Review of Systems: a careful review revealed a history of more than 20 years of joint pain accompanied by morning stiffness and both dry eyes and mouth.  The joint pain is generally symmetric, and involves her wrists, shoulders, knees, and most of the small joints of the hands. 

Physical Exam

She is a well-developed 64-inch, 140-pound middle-aged woman in no distress. She is afebrile with a comfortable breathing pattern and a respiratory rate of 16 breaths/minute, a heart rate of 85 beats/minute,  a blood pressure of 140/78 mmHg, and an oxygen saturation of 96%.  Conjunctivas are injected and scleras are anicteric.  Nasal mucosa is pink and there are no polyps.  Neck is supple and there is a well-healed tracheotomy scar. There is no lymphadenopathy at any nodal station.  Oropharyx is without erythema or exudates. Chest is clear to auscultation and normal to percussion but there is a slightly prolonged expiratory phase.  Heart is regular without murmur, rub, or gallop. Abdomen is soft and nontender without hepatosplenomegaly.  Skin is clear and has normal texture.  Her joint exam is significantly abnormal with swelling, warmth, and tenderness involving both wrists, and all second and third PIP and MCP joints.  Her knees are swollen with mild bony deformity.  There is no nailbed clubbing or lower extremity edema. There is no evidence of neurological abnormality.

Lab

Pulmonary Function Testing Spirometry:  FEV1/FVC 64%, FEV1 0.96 liters (36% predicted). (Flow-Volume Loop seen in Figure 1) Lung Volumes: TLC 75% predicted, RV 105% predicted. Diffusion Capacity: DLCO 47% predicted, DLCO/VA 62% predicted.  

Pertinent Lab Data

Alpha-1 antitrypsin level 164mg/dL (normal), Rheumatoid Factor negative, C-reactive protein 24.5 mg/L (markedly elevated), anti-cyclic citrullinated peptide 80 mg/dL (markedly elevated), complete blood count within normal limits.   

Radiographic Data

Hand radiographs:  multiple erosions of the carpal bones and distal ulna, bilaterally.  Chest radiograph – PA and lateral views – see Figures 2 and 3.   The patient was referred to a rheumatologist for further evaluation.

Figures


Figure 1. Flow Volume Loops on pulmonary function testing

Figure 2. PA chest radiograph

Figure 3. Lateral chest radiograph

Question 1

What is the likely unifying diagnosis?

References

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